@
Brit. 3. Psychiat.
130, 525—33
Psychosis in Mental
Handicap
The Tenth Blake Marsh Lecture delivered before the Royal College of Psychiatrists, February 2, 1976 By ALAN
HEATON-WAR1)
the effects of low intelligence from those of mental illness were not insoluble. Tredgold expressed the opinion that, in defectives, the commonest psychoneuroses were hysterical con version phenomena, agitated states and more or less magical obsessive-compulsive conditions; and Penrose thought that relatively mild, though often very chronic types of mental ill ness, such as obsessional neurosis, anxiety states and hysteria, probably occurred just as fre quently in defectives as in the population at large. At present it is impossible to quote even an approximate figure for the prevalence of all forms of mental illness in the total mentally handicapped population, but limited informa tion is available concerning the hospital section. Surveys of new admissions to mental handicap hospitals, and of their resident populations, have shown a prevalence of all forms of psychia tric disorder of up to 6o per cent (Leck, Gordon and McKeown, 1967; Primrose, 197k; Craft,
The literature on the purely psychiatric aspects of mental handicap is remarkably limited. In the third edition of Mayer-Gross, Slater and Roth's Clinical Psychiatry, apart from one or two brief incidental mentions elsewhere in the text, the main references consist of just one paragraph on psychoses and two short paragraphs on neuroses included in the section on ‘¿TheAssociation of Mental Subnormality with Other Syndromes'! Similarly the only reference to mental illness in mental handicap in the eighth edition of Henderson and Gilles pie's Text-Book of Psychiatry was a mere four lines on psychosis; by the ninth edition, this had grown to seven lines covering both neuroses and psychoses, but with two of those lines devoted to an admission by the authors of their own lack of knowledge. Nor can we claim that the subject of mental illness is as yet adequately dealt with in standard textbooks on mental handicap. It is surprising how little attention it has been given at the five International Conferences on the Scientific Study of Mental Deficiency held in the past fifteen years. In fact, out of more than I ,300 papers on all aspects of mental handicap less than 40 were concerned with mental illness, psychopathy or their treatment, and of these more than half were about autism or childhood psychosis and only two were specifically con cerned with psychosis in the adult mentally handicapped. In 1955, Tredgold pointed out that because of the undifferentiated state of organization of many subnormal personalities definite textbook categories of mental illness tended to be con spicuously absent among them. However, ten years later Penrose, in his 1965 Maudsley Lecture, claimed that the problems of separating
1971;
Reid,
7972).
Such
figures
reflect
both
the
admission policy of the hospitals concerned and the interests of their consultant staff in different types of psychiatric disorder; but, if one ex cludes patients whose only form of mental disorder is one of personality, manifesting itself in inadequate or antisocial behaviour, the prevalence of psychosis and neurosis combined in these surveys appears to be between 8 and 10 per
cent.
The literature concerning manic-depressive psychosis, schizophrenia, and paranoid psy chosis in adult mental defectives was well reviewed by Reid in 7972, revealing the often conflicting views expressed by various authors. Thus, Gordon (7978) noted that depression was more common than mania, whereas Duncan 525
This
One
P2TJ-R3X-5FZG
PSYCHOSIS IN MENTAL HANDICAP
526 (1936)
claimed
that
mania
was
four
times
as
common as melancholia. While Earl (1961) asserted that ‘¿sincecyclothymia does not occur in subnormal patients, they do not suffer from manic-depressive psychosis', others, including Penrose (1963) and Duncan (1936), believed it could occur in the less severe case of mental defect, but was practically unknown among idiots. Kraepelin (5896, 1902) believed that schizo phrenic psychosis beginning in the first decade could itself cause mental defect, whereas Luther (1913)
thought
that
the
occurrence
of
both
conditions in the same individual was merely a chance combination. The majority of subse quent authors, including Penrose (1963), appear
agreed
@
@
in rejecting
isolated symptoms
such as
the mannerisms and stereotypies shown by young idiots as signs of schizophrenia, as Kraepelin had suggested. Prompted by these conflicting opinions, Reid personally examined and followed up for at least six months all those mental defectives over the age of i 6 years, at Strathmartine and the Royal Dundee LilT Hospitals, considered to be suffering from mental illness and referred to him by the medical and nursing staff of those hospitals. He made a diagnosis of manic depressive psychosis in i@ female and 8 male patients, including 3 idiots, two of whom had family histories of the same illness, a diagnosis of schizophrenic psychosis in female and 8 male patients; and a diagnosis of paranoid
he ‘¿couldsubstantiate the diagnosis in 3 idiots'. On the evidence presented in his paper, the i 8 imbecile, feeble-minded and borderline de fectives certainly appeared to be suffering from affective psychoses. Most of them had only one psychotic episode during the six months' period while the patients were under Reid's personal observation, but all had had such episodes previously (Reid, personal communication). As Reid himself stated, diagnosis of this condition in idiots can be disputed; in his 3 cases it was based on prolonged study of behaviour and sleep patterns by observers who knew the patients well, and on a knowledge of the family history. In one case, there were phases of mischievous activity lasting so to 20 days, accompanied by early morning waking, self injury and irritability. The second patient had an apparently depressive spell lasting eight weeks, with refusal of food, loss of weight and very early waking from sleep. In the third patient, phases of noisy over-activity, weeping and depression, each lasting about two weeks, developed into a more prolonged state of depression, in which she behaved in a more childish way than usual, attempting to draw attention to herself by adopting unusual postures and inflicting injury on herself. In spite of the presence of positive family histories of manic-depressive psychosis, diagnosis of this condition in each of these cases appears, on this evidence, to be highly speculative. Chromo
somal abnormalities
were present in 4 of the 2!
psychosis in a further 4 females and 3 males. This represented a combined prevalence rate
patients—two
for all of these conditions among the 500 or so patients at Strathmartine of around per thousand— 12 per thousand for manic-depressive
some in the E group had a well-defined satellite. Reid reported that tricycic antidepressants and ECT appeared to be effective in most cases of depressive psychosis in the feeble-minded and
psychosis and 32 per thousand for schizophrenic psychosis (including paranoid psychosis). Reid explained that he had not undertaken a survey of the whole patient population at Strath.. martine, and these prevalence estimates there Ibre, represented a minimum and reflected the ability of staff to identify mental illness in mental defectives under their care.
Reid
stated
that
he was able
to diagnose
cases of trisomy
triple X Syndrome,
borderline
25, one
of the
and one in which a chromo
cases in his series. In a later paper,
Reid (i@7@) reported the result of a two-year double blind trial of the prophylactic effect of lithium therapy on a group of mentally de fective patients at Strathmartine Hospital, who were suffering from frequently recurring affective or behavioural changes. He found that the
manic-depressive psychosis ‘¿on the usual clinical grounds in feeble minded and borderline
number of weeks ill during the year on lithium was significantly less than the number of weeks ill during the year on the placebo; but, although
defectives and in some imbeciles',
the
and thought
number
of episodes
during
the
year
on
ALAN HEATON-WARD
lithium was less than that during the year on the placebo, the difference was not statistically significant. Reid appears to be on surer ground in his diagnosis of schizophrenic and paranoid psy choses, which he felt could be made on the usual clinical grounds in feeble-minded and borderline mental defectives and in some higher-grade imbeciles. In his opinion, the inability of most idiots to communicate verbally precluded the possibility of diagnosing schizo phrenic and paranoid psychoses in those patients. He was able to recognize hebephrenic and catatonic schizophrenia, but not simple schizophrenia, which he felt had few features to distinguish it from the effects of prolonged unstimulating hospitalization—a view which I fully share, as it seems to me that the diagnosis of simple schizophrenia in the mentally handi capped calls for the same degree of extra sensory perception which I feel must be possessed by those members of the staff who constantly report the occurrence of petit mal in patients asleep in bed at night! Among the i 2 patients with schizophrenic psychosis, the onset had been in the second or third decade, and Reid found evidence that the psychoses ran a more benign course in mental defectives, especially those in the lower grades of intelligence. The paranoid psychoses had begun in the fifth, sixth and seventh decades, and Reid noted an accompanying severe dis order of vision or hearing or both in 42 per cent of these patients. Again, there were chromosomal abnormalities in 4 patients—one case of trisomy 2!, one mosaic mongol with a 46,XX/47,XX trisomy 21, and one case of 48,XXYY syndrome, all in the paranoid psychotic group, with none in the schizophrenic psychotic group. Reid reported an apparently satisfactory response to phenothiazines in 25 per cent of patients with schizophrenic psychosis, and a reduction of agitation and irritability in those with paranoid psychoses. With the help of my consultant colleagues, Dr Joseph Jancar and Dr Ruth Walters, and of other medical and nursing staff, I carried out an investigation of psychotic mental illness occurring in the 1,251 adult mentally handi
527
capped patients in the four hospitals of the Stoke Park Group. In the case of patients at three of the hospitals—Purdown, Leigh Court and Hanham Hall—I followed Reid's pattern of investigating only those cases referred to me by Dr Jancar and Dr Walters as showing signs of psychoses. In the case of Stoke Park Hospital, I personally reviewed the case history of each of the 363 adult female and 65 male patients under my care, and of the 37 female and 19 male patients in Stoke Park under the care of the other two consultants. Difficulties start immediately with the diag nostic criteria of psychosis in general and of the commonly described clinical types of psychosis in particular. The Encyclopaedia of Psychiatry defines psychosis as ‘¿Mentalillness which is severe, produces conspicuously disordered beha viour, cannot be understood as an extension or exaggeration of ordinary experience and whose subject is without insight'. On this basis, all those of our profoundly retarded patients (IQ approximately 20 or less) and some of our severely retarded patients (IQ approxi mately 20—35),who have no intelligible speech, in whom emotional lability, noisy outbursts, continuous or periodic disorganized, purposeless activity, including aggression, destructiveness and self mutilation are the rule, could be con sidered psychotic. However, the generally accepted criterion for distinguishing between a neurosis and a psychosis is the presence of insight and retention of a sense of reality in the former and their absence in the latter, and it appears to me that, for a clinician to be able to deter mine with certainty the presence or absence of insight, a patient must have at least some form of intelligible communication, if not intelligible verbal speech. Anyone undertaking such a survey will quickly realize how dependent he is on the quality of the clinical notes by medical staff and on the accurate daily recording of observations on the patient's behaviour by the nursing staff. Without such information, accurate retro spective diagnosis is made far more difficult, if not impossible. Not that diagnosis will always be easy even where the most accurate notes are available, and the often protean presentation of mental illness in the mentally handicapped
528
PSYCHOSIS
IN MENTAL
makes the need to keep the diagnosis and treat ment under constant review just as great as the need for an ongoing rather than a once-and-for all assessment of their underlying handicap. Mental illness which appears to be developing into a classical case of one particular psychosis may often prove to be an undoubted case of another when reviewed over a period. Some of the diagnostic difficulty arises from the misuse of psychiatric terms by nursing and other staff; for example, the use of the term ‘¿mania'to describe the restless over-activity associated with what is in fact an agitated depression may lead not only to inaccurate diagnosis but to the prescribing of completely inappropriate drugs which intensify the depression. Again, it may be impossible from the notes available to establish even an approximate date of onset of a psychosis, particularly of the affective variety, and the absence of information on the highest pre-psychotic IQ in patients admitted to hospital some time after their psychosis has started tends to invalidate esti mates of the prevalence of the various psychoses at different levels of intelligence. In spite of these difficulties, I felt I could make a confident diagnosis of affective psychosis in 8 male and 8 female patients, and that all but one of them (a female aged 83 at Stoke Park with a senile depression) satisfied my criterion for a diagnosis of manic-depressive psychosis in that they had a history of recurring episodes of depression or mania or both. These 15 patients represent a prevalence rate of I 2 per thousand for the Stoke Park Group (13
per
thousand
for males
and
i i per
thousand
for females)—a figure identical with Reid's figure for Strathmartine. However, the varia tion in prevalence rates for each of the four hospitals suggests that it is very doubtful whether this represents a true picture for the Group as a whole. The prevalence rate for Stoke Park Hos pital itself is 19 per thousand, for Purdown Hos pital io per thousand, for Leigh Court Hospital 6 per thousand, and for Hanham Hall Hospital 5 per thousand. It is possible to account for part of the variation by the different admis sion policies followed at each of the hospitals in the past, but I think an equally important factor may be the different methods of identi
HANDICAP
fying patients with psychotic illness adopted by myself at Stoke Park Hospital, and by my colleagues at the other three hospitals. It would, I think, be surprising if a survey of the case histories of every patient in any hospital did not identify more patients with a history of mental illness at some time since admission than a survey based merely on the identification of patients with signs of mental illness currently active or active in the fairly recent past. I suspect, therefore, that the true prevalence rates of manic-depressive psychosis for the Stoke Park Group as a whole are almost certainly higher than those I have quoted. Five male and 5 female patients had both manic and depressive episodes, and 3 male and 2 female
patients
had
depressive
episodes
alone.
Accurate family histories were rarely available, and in only 3 of the cases of manic-depressive psychosis was a positive family history of psychotic illness obtained. The highest recorded IQ's of the manic depressive group were within the range 39—74, in the case of males, and 47—63in the case of females. The age of onset was in the third, fourth or fifth decade in males (with the exception of one patient in whom the onset was at the age of i 5) and
in the
fourth,
fifth
and
sixth
decades
in
females. The longest interval between episodes was 6 years in males and 13 years in females. The ambiguity of the case notes made it difficult to be precise about the shortest interval between episodes, but this appeared in some cases to be as short as a month in both males and females. For the same reason, it was sometimes difficult to establish the exact number of manic or depressive episodes which had occurred in any individual. Noisy, boisterous, interfering over-activity by both day and night, with obscene language and aggression towards patients and staff, was a frequent occurrence during the manic phase. In their manic phases, one male patient ex pressed grandiose delusions of supernatural powers, and another indulged in bizarre sexual activity. Paranoid features occurred during both the manic and depressive phases, but more commonly during the latter, in which delusions of bodily malfunction were also common. Several depressed patients expressed ideas of
ALAN
HEATON-WARD
guilt and unworthiness and made suicidal threats, but none attempted this. A chromosome abnormality was found in one patient only—a male with a 48,XXYY Syndrome, who was also diabetic. The patient in whose case the onset was at the age of 15 years was diagnosed as suffering from intermittent porphyria at the age of 3!. For four years before this he had been receiving lithium therapy, and he has remained free from manic-depressive episodes ever since. None of the other patients in the manic-depressive group showed signs of any of the syndromes associated with mental handicap—they were, in fact, noteworthy for their freedom from any obvious physical abnormality. All patients had been treated with a variety of psychotropic drugs, some with ECT, and some with both. Chlorpromazine or haloperidol were usually effective in controlling the manic episodes, but the tricycic antidepressants were less predictable in their effect on the depressive episodes. Five of the patients were receiving prophylactic lithium; of these one male and one female patient appeared much more stable on this drug, but the other three showed no obvious benefit from it, although it was doubtful whether accurate prophylactic levels had always been maintained. Two male and four female patients had received one or more courses of ECT during depressive phases. The typical features of manic-depressive psychosis are well shown in the case of Florrie, now aged 74, whose highest recorded IQwas 47. Her psychosis started with a depressive attack nearly fifteen years ago at the age of 59, and she had her first manic episode nine years later. Since a further attack of mania two years ago her manic and depressive episodes have tended to alternate, with only brief intervals of little over a month between them. In her manic phase her mood is elevated and she has pressure of speech, with ifights of ideas influenced by events in her environment. This continues for several days until haloperidol takes effect. In her depressed phase she expresses marked paranoid ideas concerning patients and staff. Delusions of bodily malfunction in recurrent depressive attacks are well shown by Mary, now aged 6i, whose highest recorded IQ was 6o.
529
Her first attack of depression occurred at the age of@, with the development, ten years later, of delusions of inability to swallow. Treatment with antidepressant drugs was further compli cated by her conviction that they were causing her to stamp her feet, when in fact this was a manifestation of her agitation rather than of drug-induced akathisia. These delusions have reappeared each time her depressive illness has returned. My survey revealed a number of patients for whom I had doubts about making a definite diagnosis of affective psychosis. These included two severely retarded females aged 42 and 45 at Stoke Park Hospital, who showed persistently boisterous, over-active, noisy and occasionally aggressive and destructive behaviour, with a predominantly elated mood suggestive of chronic mania. In the elder patient, the highest re corded IQwas 29, and in the younger—22, but the verbal facility of the latter suggested that her IQ must have been higher before her admission 13 years previously. Her behaviour had become more unmanageable on lithium, and both she and the other patient had shown only a minimal beneficial response to a variety of psychotropic drugs. A male patient at Stoke Park aged 32, with limited but intelligible speech, and with a highest recorded IQ of only 20,
had
a number
of features
in
common
with
the two female patients and presented a similar diagnostic problem. I felt able to make a confident diagnosis of schizophrenic illness on the basis of the usually accepted clinical criteria in the case of 15 males and 27 females—an even more marked reversal of the usually accepted sex distribution than that for manic-depressive psychosis, which I am at present unable to explain. These figures represent a prevalence rate for the Stoke Park Group as a whole of 34 per thousand (24 per thousand for males and 40 per thousand for females), very similar to Reid's figure for Strathmartine, but again there are wide variations between the prevalence rate for Stoke Park and that for each of the other three hospitals—Stoke Park 50 per thousand, Pur down 15 per thousand Leigh Court 53 per thousand and Hanham Hall 15 per thousand. The more obvious mental disturbance in
530
PSYCHOSIS IN MENTAL
schizophrenia, and its more or less ever-present nature, makes it less likely to be overlooked by medical or nursing staff than an episodic illness like manic-depressive psychosis, in which the last attack may have occurred some years before. Assuming, therefore, that my diagnosis was correct in each case, I think that the true prevalence of schizophrenic illness in the Stoke Park Group and at Strathmartine Hospital is likely to be little, if at all, higher than the figures quoted by Reid and myself. A diagnosis of hebephrenic schizophrenia was made for 10 males and 13 females. The age at onset appeared to be in the second or third decade in both sexes. In 2 male and 4 female cases the onset had been before the age of i6 years. The highest recorded IQ was in the range of 42—69in males, with the exception of one patient with an IQ of approximately 20, and in females it was in the range of 28—70, except that one patient had an IQ of 23; this patient had, however, been reported earlier as being able to read. This again illustrates the difficulty of establishing an accurate pre psychotic IQ in a case of schizophrenia seen for the first time a considerable period after the onset of the disorder. A case in point is that of Christopher, who was almost
@
15 when
he came to
Purdown in 1959 with a highest recorded IQof 58, but who is now able to express concepts which suggest that his IQ must previously have been considerably higher; it is now 51. A positive family history of psychosis was obtained in only male and 6 female patients. The most interesting concerned Jimmy, whose maternal grandmother and maternal uncle had suffered from serious psychotic illnesses, and whose younger sister, Jackie, was admitted to Stoke Park Hospital two years after her brother, with profound mental retardation but with nothing to suggest a psychosis. On admission, Jimmy's IQ was 42, whereas Jackie was only at the 6—cjmonth stage of development on the Griffiths Scale when she came to us. It is interesting to speculate that, whatever the nature of the schizophrenic process may eventually prove to be, its presence at birth may, as in the case also of the inborn errors of metabolism, inhibit almost completely normal mental development so as to produce a condi
HANDICAP
lion of profound mental retardation, indis tinguishable, as in Jackie's case, from that of many others with a similar degree of mental handicap. The frequent occurrence of autistic and non-specific psychotic features, in children with phenylketonuria and other inborn meta bolic errors, seems to add support to such speculation. Five male and 4 female patients were diag nosed as suffering from catatonic schizophrenia, on the basis of recurrent periods of extreme psychotic excitement and catatonic stupor with flexibilitas cerea, with intervals of more or less normal behaviour in between. I felt able to make a diagnosis of paranoid psychosis in i z female cases but in no males. Three of the patients had systematized delusions. The age of onset ranged from the fourth to the seventh decade, but in 5 cases it was in the fifth decade, which raises the possibility of the menopause as an important aetiological factor which might partially explain the prepon derance of female cases. Similarly, the highest recorded IQ showed a wide variation from 26 to 72, but again the patient with the IQ of 26 has a verbal facility which suggests a previously higher IQ, and the possibility that she would be more correctly diagnosed as suffering from hebephrenic schizophrenia, in spite of the absence of any definite evidence in her history of psychosis before the fifth decade. In 3 patients with paranoid psychosis there were signs of superimposed senile dementia. One patient in the paranoid psychosis group had an XO/XX chromosome mosaic comple ment. She was also deaf and blind from retinitis pigmentosa, and had suffered from oro-facial dyskinesia since 5960. Deafness was also present in another patient in this group (who suffered from congenital syphilis also), and in one male patient in the catatonic group. Epileptic fits had occurred in one male and in 2 females in the hebephrenic and one male in the cata tonic group. One male in the hebephrenic group suffered from congenital syphilis also, and another male in the same group was diabetic. Christopher suffered from eczema, and Jimmy from psoriasis, and in both cases the condition of their skin became worse during acute phases
ALAN HEATON-WARD
of their hebephrenic illness. Both were referred to by DrJancar in his Blake Marsh Lecture two years ago, when he reminded us of the greatly increased risk of further severe and irreversible brain damage that patients with such disorders run from vaccination. As mentioned previously, Reid found some evidence that the schizophrenic psychoses appeared to run a more benign course in the mentally handicapped than in patients with higher intelligence. Unfortunately, the results of up-to-date psychological assessment were not available in all cases in my survey, but the most recent assessment showed a fall in IQ of over 20
per
cent
in
5
patients
in
the
hebephrenic
group and in one patient in the catatonic group and one in the paranoid group, apart from the 3 patients already referred to as showing signs of superimposed senile dementia. Patients in both the schizophrenic psychosis and paranoid psychosis groups had been treated with a variety of phenothiazine, butyrophenone and other tranquillizers, with at least temporary symptomatic relief in most cases. Four hebe phrenic patients and one with paranoid psy chosis each received one or more courses of ECT. One male hebephrenic patient had received 33 deep insulin comas before his admission to Stoke Park, and had on one occasion developed irreversible coma and had been unconscious for two days. There were a number of other patients with a history of isolated paranoid behaviour, whose condition, I felt, did not justify a diagnosis of paranoid
psychosis.
I found no difficulty in identifying patients with pseudo-psychosis, whose apparent delu sions were more like childhood fantasies, and I did not include them in either the schizo phrenic psychosis or the paranoid psychosis group. Among these patients were Sidney, with an IQ of only 33, who could neither read nor write and who had been with us ever since he was admitted 52 years ago at the age of 14. He gave an enthusiastic, if somewhat im probable, account of his service in each of the armed forces in turn, claiming to have captained a ship in the Royal Navy. Another was Bobby, a very cheerful, sociable mongol out-patient aged 31, who had caused some anxiety at the
53' training centre which he attended by recounting his conversations with ‘¿his boys'; these turned out to be the television characters Randall and Hopkirk whom he had incorporated into his child-like fantasies and with whom he held imaginary conversations each day. My review of the histories of each of the adult patients at Stoke Park revealed a number who had one or more confusional episodes, most frequently associated with a febrile illness, but in one case with the development of classical signs of pellagra which was successfully treated with nicotinamide. There are at present 129 patients in the Stoke Park Group aged 70 and over, including 24 over
8o
and
one
I was particularly and Richards
over
90.
During
my
survey,
struck as both Reid (@@‘@)
(i@7@) had
been earlier
by how
few appeared to be showing the more obvious signs of senile dementia, such as confusion, memory loss, reversal of sleep patterns, wander ing, and deterioration in personal habits and hygiene. This was particularly striking in our 8o-plus group of old ladies, whose IQ's were mostly in the 40 to 50 range. It would seem that, contrary to my earlier impression, the signs of senile dementia do not necessarily appear earlier in the moderately and mildly retarded than in the general population, but perhaps this is true only of mentally handicapped patients cared for in an environment in which they are protected from the strains and stresses of everyday life in the community and from the self-neglect to which old people living on their own are prone. As a result of this survey, I found myself wondering why psychosis occurs in some mentally handicapped patients and not in others of the same level of intelligence, and whether the same aetiological factors apply to them
as to people
of normal
intelligence.
Of
all the autosomal chromosomal abnormalities associated with mental handicap, trisomy 21 is the one in which psychoses have most fre quently been reported by various authors, including Roith (1961, psychotic depression), Earl (@3@) and Rollin, (1946, catatonic psy chosis) and Payne (1968, paranoid psychosis). There is a great excess of cases of trisomy 21 over the number of all the other known chromo
PSYCHOSIS IN MENTAL HANDICAP
532
some abnormalities in the mentally handi capped combined, and there is at present no clear evidence that the prevalence of psychosis in mongols is, in fact, higher than that in sub jects with other chromosomal abnormalities or in the general population. However, several authors, including Reid (i@7@) and Richards (1974),
have
commented
on
the
particular
lia
bility of patients with Down's syndrome to under go premature ageing and dementia. We have one such patient, now aged 64 at Stoke Park, who has shown progressive mental deterioration of a senile type over the past six years, im mediately following an operation for cataract. On the other hand, psychoses have been reported in a number of mentally handicapped patients with a variety of abnormalities of sex chromosomes, in particular in males with extra X chromosomes. As Professor Forssman said in his 5970 Blake Marsh Lecture, ‘¿Itmay be considered established thatmen havingmore than one X chromosome are more subject to func tional psychosis than are men with only one Y chromosome.' It seems possible that sex has, in fact, more to do with mental illness than even Freud realized! In 1972, Mendlewicz and his colleagues re ported 7 families in New York in whose male members the liability to develop manic depressive psychosis seemed to go with the presence of the X-linked colour blindness gene. More recently, interest has been revived in Renpenning's syndrome, first reported in Canada in 1962 and 1963, since evidence has accumulated that this X-linked form of mental retardation is relatively common. It has been claimed by Turner (i@7i) that as many as io per cent of all mentally retarded males in the 30—35IQ range are in fact cases of Ren penning's syndrome. The physical appearance of affected persons is normal, and to date no constant biochemical abnormalities have been identified in them. As far as I know, there are no reports of the association of colour blindness or manic-depressive psychosis with Ren penning's syndrome, and I think that this would provide a fertile field for research—it would be fascinating if this association could be demonstrated and a link established between Mendlewicz's and Renpenning's cases.
The present Chairman of the Mental Defi ciency Section, Dr Valerie Cowie (1968), and other authors, including Stern (1968) and Winokur (1974), have discussed the association between a number of inborn errors of meta bolism, such as cystathionuria, homocystinuria and Hartnup disorder, and the psychoses, including schizophrenia, and the possibility of their giving clues to the biochemical mecha nisms of thought disorder—yet another ob viously fertile field for research. I feel very strongly that the staff of a mental handicap hospital should be capable of con tinning to care themselves for all their patients who develop mental illnesses, but I agree with Reid that junior staff have often had little, if any, psychiatric training and that training in clinical psychiatry, psychopharmacology and the techniques of ECT are necessary aspects of the training programmes for both medical and nursing staff. I am very concerned at the attitude of those of our medical and nursing colleagues in the mental handicap field who take the view that patients who develop a superimposed mental illness should be transferred for treatment to a mental illness hospital. In so doing, I think that our medical colleagues are calling in to question any claim they may have to be considered psychiatrists, and are reinforcing the case of those who deny the existence of the specialty of mental handicap to which Blake Marsh devoted his working life; and similarly our nursing colleagues who take this view are dangerously ‘¿jay walking' and are strengthening the case of those who would abolish the present register of nurses of the mentally subnormal and hand over the nursing care of our patients to a new caring profession. I hope I have succeeded in my aim of demonstrating that mental handicap does belong to psychiatry and that it contains a wealth of psychiatric material, as yet not fully explored, which may provide essential clues to our understanding of psychotic illness through out the full range of human intelligence. REFERENcES Cit@rr, M. (i@7i) A North Wales experiment in sub normality care. British Journal of Psychiatry, i i8, 199—206.
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Evidence for X-linkage in the transmission of manic depressive illness. Journal of the American Medical Association, 222, 1624. NAYLOR, G. J., DONALD, J. M., LE PoivanlAN, D. & REm, A. H. (,@7i@)A double-blind trial of long-term lithium therapy on mental defectives. British Journal
of Psychiatry,124, 52-7.
Alan Heaton-Ward, BSi6
(1963)
Journal
of
Biology
of
Mental
Defect,
3rd
The
contribution
of
(197
to 1968
i)
A
survey
of
a subnormality to 31st
mental
deficiency
British Journal
of Psychiatry,
500
consecutive
hospital
December
1970.
from
British
1st
Journal
—¿
55,
205—18.
(1974)
Dementia
in
ageing
mental
defectives.
Journal
RENPENNINO,
H., T.
GERRARD, (1962)
J.
W.,
Familial
ZALESKI,
sex-linked
Q.
A.
&
retardation.
Canadian Medical Association Journal, 87, 954. RICHARDS,
B.
W.
(ig7@)
Old
age
and
the
mentally
retarded. In Modern Perspectives in Psychogeriatrics. Edinburgh: Oliver and Reed. Rorrii, A. I. (1961) Psychotic depression in a mongol. Journal of Mental Subnormal4y, 7,45-7. RoUIN, H. R. (1946) Personality in mongolism, with special reference to catatonic psychosis. American
Journal of Mental Deficiency,51, 219-37. STERN,
J.
&
Cowix,
V.
(1968)
Inherited
abnormalities
affecting the nervous system—genetic and psychiatric aspects. Proceedings of the Biochemical Society. Biochemical Journal, iii, No. 3, 9—10.
TREDOOLD,R. F. & SODDY,K. (1956) Textbookof Mental Deficiency, 9th edition. TURNER,
G.,
TURNER,
B.
London. & CouxNs,
E.
(ig7i)
X-linked
mental retardation without physical abnormality Renpenning's Syndrome. Developmental Medicine and Child Xeurology, 13, 71. WINOKUR,
B.
(i@7i@)
Subnormality
and
its
relation
to
psychiatry. Lancet, ii, 270—2.
M.B., F.R.C.Psych., Consultant Psychiatrist, Stoke Park Hospital, Stapleton, Bristol
iQU
(Received 9 March;
The
to psychiatry.
TABATA,
British Journal of Psychiatry, 126, 105—13. (i8g6)
subnormal.
of Mental DeficiencyResearch,iS, 15—23.
Journal of Insanity, 75, 489-99.
LECK,
g66)
No.
Psychoses
psychotic
of Mental Subnormality, 27, 25—8. REm, A. H. (1972) Psychoses in adult mental defectives: I. Manic depressive psychosis; II. Schizophrenia and paranoid psychoses. British Journal of Psychiatry, u@o,
353—63.
GORDON, A. (1918)
KRAEPELIN,
(i
January
London.
@ionzits (1972) London: Roche Products Ltd.
JANCAR, J.
S.
admissions
ENCYCLOPAEDIA
117,
L.
112, 747—55. PRIMROSE, D. A.
231—53. —¿
The
edition, pp 263, 264. London.
manic
Science, 82,
(ig68)
research
C.
of idiocy.
Mental
R.
Mental Subnormality, 14, 25—34.
hospital.
Journal of Xeurologvand Psychopathology,i6, 225—38. —¿
PAYNE,
533
revised 21 June 1976)
Brit. 3. Psychiat.
130, 525—33
Psychosis in Mental
Handicap
The Tenth Blake Marsh Lecture delivered before the Royal College of Psychiatrists, February 2, 1976 By ALAN
HEATON-WAR1)
the effects of low intelligence from those of mental illness were not insoluble. Tredgold expressed the opinion that, in defectives, the commonest psychoneuroses were hysterical con version phenomena, agitated states and more or less magical obsessive-compulsive conditions; and Penrose thought that relatively mild, though often very chronic types of mental ill ness, such as obsessional neurosis, anxiety states and hysteria, probably occurred just as fre quently in defectives as in the population at large. At present it is impossible to quote even an approximate figure for the prevalence of all forms of mental illness in the total mentally handicapped population, but limited informa tion is available concerning the hospital section. Surveys of new admissions to mental handicap hospitals, and of their resident populations, have shown a prevalence of all forms of psychia tric disorder of up to 6o per cent (Leck, Gordon and McKeown, 1967; Primrose, 197k; Craft,
The literature on the purely psychiatric aspects of mental handicap is remarkably limited. In the third edition of Mayer-Gross, Slater and Roth's Clinical Psychiatry, apart from one or two brief incidental mentions elsewhere in the text, the main references consist of just one paragraph on psychoses and two short paragraphs on neuroses included in the section on ‘¿TheAssociation of Mental Subnormality with Other Syndromes'! Similarly the only reference to mental illness in mental handicap in the eighth edition of Henderson and Gilles pie's Text-Book of Psychiatry was a mere four lines on psychosis; by the ninth edition, this had grown to seven lines covering both neuroses and psychoses, but with two of those lines devoted to an admission by the authors of their own lack of knowledge. Nor can we claim that the subject of mental illness is as yet adequately dealt with in standard textbooks on mental handicap. It is surprising how little attention it has been given at the five International Conferences on the Scientific Study of Mental Deficiency held in the past fifteen years. In fact, out of more than I ,300 papers on all aspects of mental handicap less than 40 were concerned with mental illness, psychopathy or their treatment, and of these more than half were about autism or childhood psychosis and only two were specifically con cerned with psychosis in the adult mentally handicapped. In 1955, Tredgold pointed out that because of the undifferentiated state of organization of many subnormal personalities definite textbook categories of mental illness tended to be con spicuously absent among them. However, ten years later Penrose, in his 1965 Maudsley Lecture, claimed that the problems of separating
1971;
Reid,
7972).
Such
figures
reflect
both
the
admission policy of the hospitals concerned and the interests of their consultant staff in different types of psychiatric disorder; but, if one ex cludes patients whose only form of mental disorder is one of personality, manifesting itself in inadequate or antisocial behaviour, the prevalence of psychosis and neurosis combined in these surveys appears to be between 8 and 10 per
cent.
The literature concerning manic-depressive psychosis, schizophrenia, and paranoid psy chosis in adult mental defectives was well reviewed by Reid in 7972, revealing the often conflicting views expressed by various authors. Thus, Gordon (7978) noted that depression was more common than mania, whereas Duncan 525
This
One
P2TJ-R3X-5FZG
PSYCHOSIS IN MENTAL HANDICAP
526 (1936)
claimed
that
mania
was
four
times
as
common as melancholia. While Earl (1961) asserted that ‘¿sincecyclothymia does not occur in subnormal patients, they do not suffer from manic-depressive psychosis', others, including Penrose (1963) and Duncan (1936), believed it could occur in the less severe case of mental defect, but was practically unknown among idiots. Kraepelin (5896, 1902) believed that schizo phrenic psychosis beginning in the first decade could itself cause mental defect, whereas Luther (1913)
thought
that
the
occurrence
of
both
conditions in the same individual was merely a chance combination. The majority of subse quent authors, including Penrose (1963), appear
agreed
@
@
in rejecting
isolated symptoms
such as
the mannerisms and stereotypies shown by young idiots as signs of schizophrenia, as Kraepelin had suggested. Prompted by these conflicting opinions, Reid personally examined and followed up for at least six months all those mental defectives over the age of i 6 years, at Strathmartine and the Royal Dundee LilT Hospitals, considered to be suffering from mental illness and referred to him by the medical and nursing staff of those hospitals. He made a diagnosis of manic depressive psychosis in i@ female and 8 male patients, including 3 idiots, two of whom had family histories of the same illness, a diagnosis of schizophrenic psychosis in female and 8 male patients; and a diagnosis of paranoid
he ‘¿couldsubstantiate the diagnosis in 3 idiots'. On the evidence presented in his paper, the i 8 imbecile, feeble-minded and borderline de fectives certainly appeared to be suffering from affective psychoses. Most of them had only one psychotic episode during the six months' period while the patients were under Reid's personal observation, but all had had such episodes previously (Reid, personal communication). As Reid himself stated, diagnosis of this condition in idiots can be disputed; in his 3 cases it was based on prolonged study of behaviour and sleep patterns by observers who knew the patients well, and on a knowledge of the family history. In one case, there were phases of mischievous activity lasting so to 20 days, accompanied by early morning waking, self injury and irritability. The second patient had an apparently depressive spell lasting eight weeks, with refusal of food, loss of weight and very early waking from sleep. In the third patient, phases of noisy over-activity, weeping and depression, each lasting about two weeks, developed into a more prolonged state of depression, in which she behaved in a more childish way than usual, attempting to draw attention to herself by adopting unusual postures and inflicting injury on herself. In spite of the presence of positive family histories of manic-depressive psychosis, diagnosis of this condition in each of these cases appears, on this evidence, to be highly speculative. Chromo
somal abnormalities
were present in 4 of the 2!
psychosis in a further 4 females and 3 males. This represented a combined prevalence rate
patients—two
for all of these conditions among the 500 or so patients at Strathmartine of around per thousand— 12 per thousand for manic-depressive
some in the E group had a well-defined satellite. Reid reported that tricycic antidepressants and ECT appeared to be effective in most cases of depressive psychosis in the feeble-minded and
psychosis and 32 per thousand for schizophrenic psychosis (including paranoid psychosis). Reid explained that he had not undertaken a survey of the whole patient population at Strath.. martine, and these prevalence estimates there Ibre, represented a minimum and reflected the ability of staff to identify mental illness in mental defectives under their care.
Reid
stated
that
he was able
to diagnose
cases of trisomy
triple X Syndrome,
borderline
25, one
of the
and one in which a chromo
cases in his series. In a later paper,
Reid (i@7@) reported the result of a two-year double blind trial of the prophylactic effect of lithium therapy on a group of mentally de fective patients at Strathmartine Hospital, who were suffering from frequently recurring affective or behavioural changes. He found that the
manic-depressive psychosis ‘¿on the usual clinical grounds in feeble minded and borderline
number of weeks ill during the year on lithium was significantly less than the number of weeks ill during the year on the placebo; but, although
defectives and in some imbeciles',
the
and thought
number
of episodes
during
the
year
on
ALAN HEATON-WARD
lithium was less than that during the year on the placebo, the difference was not statistically significant. Reid appears to be on surer ground in his diagnosis of schizophrenic and paranoid psy choses, which he felt could be made on the usual clinical grounds in feeble-minded and borderline mental defectives and in some higher-grade imbeciles. In his opinion, the inability of most idiots to communicate verbally precluded the possibility of diagnosing schizo phrenic and paranoid psychoses in those patients. He was able to recognize hebephrenic and catatonic schizophrenia, but not simple schizophrenia, which he felt had few features to distinguish it from the effects of prolonged unstimulating hospitalization—a view which I fully share, as it seems to me that the diagnosis of simple schizophrenia in the mentally handi capped calls for the same degree of extra sensory perception which I feel must be possessed by those members of the staff who constantly report the occurrence of petit mal in patients asleep in bed at night! Among the i 2 patients with schizophrenic psychosis, the onset had been in the second or third decade, and Reid found evidence that the psychoses ran a more benign course in mental defectives, especially those in the lower grades of intelligence. The paranoid psychoses had begun in the fifth, sixth and seventh decades, and Reid noted an accompanying severe dis order of vision or hearing or both in 42 per cent of these patients. Again, there were chromosomal abnormalities in 4 patients—one case of trisomy 2!, one mosaic mongol with a 46,XX/47,XX trisomy 21, and one case of 48,XXYY syndrome, all in the paranoid psychotic group, with none in the schizophrenic psychotic group. Reid reported an apparently satisfactory response to phenothiazines in 25 per cent of patients with schizophrenic psychosis, and a reduction of agitation and irritability in those with paranoid psychoses. With the help of my consultant colleagues, Dr Joseph Jancar and Dr Ruth Walters, and of other medical and nursing staff, I carried out an investigation of psychotic mental illness occurring in the 1,251 adult mentally handi
527
capped patients in the four hospitals of the Stoke Park Group. In the case of patients at three of the hospitals—Purdown, Leigh Court and Hanham Hall—I followed Reid's pattern of investigating only those cases referred to me by Dr Jancar and Dr Walters as showing signs of psychoses. In the case of Stoke Park Hospital, I personally reviewed the case history of each of the 363 adult female and 65 male patients under my care, and of the 37 female and 19 male patients in Stoke Park under the care of the other two consultants. Difficulties start immediately with the diag nostic criteria of psychosis in general and of the commonly described clinical types of psychosis in particular. The Encyclopaedia of Psychiatry defines psychosis as ‘¿Mentalillness which is severe, produces conspicuously disordered beha viour, cannot be understood as an extension or exaggeration of ordinary experience and whose subject is without insight'. On this basis, all those of our profoundly retarded patients (IQ approximately 20 or less) and some of our severely retarded patients (IQ approxi mately 20—35),who have no intelligible speech, in whom emotional lability, noisy outbursts, continuous or periodic disorganized, purposeless activity, including aggression, destructiveness and self mutilation are the rule, could be con sidered psychotic. However, the generally accepted criterion for distinguishing between a neurosis and a psychosis is the presence of insight and retention of a sense of reality in the former and their absence in the latter, and it appears to me that, for a clinician to be able to deter mine with certainty the presence or absence of insight, a patient must have at least some form of intelligible communication, if not intelligible verbal speech. Anyone undertaking such a survey will quickly realize how dependent he is on the quality of the clinical notes by medical staff and on the accurate daily recording of observations on the patient's behaviour by the nursing staff. Without such information, accurate retro spective diagnosis is made far more difficult, if not impossible. Not that diagnosis will always be easy even where the most accurate notes are available, and the often protean presentation of mental illness in the mentally handicapped
528
PSYCHOSIS
IN MENTAL
makes the need to keep the diagnosis and treat ment under constant review just as great as the need for an ongoing rather than a once-and-for all assessment of their underlying handicap. Mental illness which appears to be developing into a classical case of one particular psychosis may often prove to be an undoubted case of another when reviewed over a period. Some of the diagnostic difficulty arises from the misuse of psychiatric terms by nursing and other staff; for example, the use of the term ‘¿mania'to describe the restless over-activity associated with what is in fact an agitated depression may lead not only to inaccurate diagnosis but to the prescribing of completely inappropriate drugs which intensify the depression. Again, it may be impossible from the notes available to establish even an approximate date of onset of a psychosis, particularly of the affective variety, and the absence of information on the highest pre-psychotic IQ in patients admitted to hospital some time after their psychosis has started tends to invalidate esti mates of the prevalence of the various psychoses at different levels of intelligence. In spite of these difficulties, I felt I could make a confident diagnosis of affective psychosis in 8 male and 8 female patients, and that all but one of them (a female aged 83 at Stoke Park with a senile depression) satisfied my criterion for a diagnosis of manic-depressive psychosis in that they had a history of recurring episodes of depression or mania or both. These 15 patients represent a prevalence rate of I 2 per thousand for the Stoke Park Group (13
per
thousand
for males
and
i i per
thousand
for females)—a figure identical with Reid's figure for Strathmartine. However, the varia tion in prevalence rates for each of the four hospitals suggests that it is very doubtful whether this represents a true picture for the Group as a whole. The prevalence rate for Stoke Park Hos pital itself is 19 per thousand, for Purdown Hos pital io per thousand, for Leigh Court Hospital 6 per thousand, and for Hanham Hall Hospital 5 per thousand. It is possible to account for part of the variation by the different admis sion policies followed at each of the hospitals in the past, but I think an equally important factor may be the different methods of identi
HANDICAP
fying patients with psychotic illness adopted by myself at Stoke Park Hospital, and by my colleagues at the other three hospitals. It would, I think, be surprising if a survey of the case histories of every patient in any hospital did not identify more patients with a history of mental illness at some time since admission than a survey based merely on the identification of patients with signs of mental illness currently active or active in the fairly recent past. I suspect, therefore, that the true prevalence rates of manic-depressive psychosis for the Stoke Park Group as a whole are almost certainly higher than those I have quoted. Five male and 5 female patients had both manic and depressive episodes, and 3 male and 2 female
patients
had
depressive
episodes
alone.
Accurate family histories were rarely available, and in only 3 of the cases of manic-depressive psychosis was a positive family history of psychotic illness obtained. The highest recorded IQ's of the manic depressive group were within the range 39—74, in the case of males, and 47—63in the case of females. The age of onset was in the third, fourth or fifth decade in males (with the exception of one patient in whom the onset was at the age of i 5) and
in the
fourth,
fifth
and
sixth
decades
in
females. The longest interval between episodes was 6 years in males and 13 years in females. The ambiguity of the case notes made it difficult to be precise about the shortest interval between episodes, but this appeared in some cases to be as short as a month in both males and females. For the same reason, it was sometimes difficult to establish the exact number of manic or depressive episodes which had occurred in any individual. Noisy, boisterous, interfering over-activity by both day and night, with obscene language and aggression towards patients and staff, was a frequent occurrence during the manic phase. In their manic phases, one male patient ex pressed grandiose delusions of supernatural powers, and another indulged in bizarre sexual activity. Paranoid features occurred during both the manic and depressive phases, but more commonly during the latter, in which delusions of bodily malfunction were also common. Several depressed patients expressed ideas of
ALAN
HEATON-WARD
guilt and unworthiness and made suicidal threats, but none attempted this. A chromosome abnormality was found in one patient only—a male with a 48,XXYY Syndrome, who was also diabetic. The patient in whose case the onset was at the age of 15 years was diagnosed as suffering from intermittent porphyria at the age of 3!. For four years before this he had been receiving lithium therapy, and he has remained free from manic-depressive episodes ever since. None of the other patients in the manic-depressive group showed signs of any of the syndromes associated with mental handicap—they were, in fact, noteworthy for their freedom from any obvious physical abnormality. All patients had been treated with a variety of psychotropic drugs, some with ECT, and some with both. Chlorpromazine or haloperidol were usually effective in controlling the manic episodes, but the tricycic antidepressants were less predictable in their effect on the depressive episodes. Five of the patients were receiving prophylactic lithium; of these one male and one female patient appeared much more stable on this drug, but the other three showed no obvious benefit from it, although it was doubtful whether accurate prophylactic levels had always been maintained. Two male and four female patients had received one or more courses of ECT during depressive phases. The typical features of manic-depressive psychosis are well shown in the case of Florrie, now aged 74, whose highest recorded IQwas 47. Her psychosis started with a depressive attack nearly fifteen years ago at the age of 59, and she had her first manic episode nine years later. Since a further attack of mania two years ago her manic and depressive episodes have tended to alternate, with only brief intervals of little over a month between them. In her manic phase her mood is elevated and she has pressure of speech, with ifights of ideas influenced by events in her environment. This continues for several days until haloperidol takes effect. In her depressed phase she expresses marked paranoid ideas concerning patients and staff. Delusions of bodily malfunction in recurrent depressive attacks are well shown by Mary, now aged 6i, whose highest recorded IQ was 6o.
529
Her first attack of depression occurred at the age of@, with the development, ten years later, of delusions of inability to swallow. Treatment with antidepressant drugs was further compli cated by her conviction that they were causing her to stamp her feet, when in fact this was a manifestation of her agitation rather than of drug-induced akathisia. These delusions have reappeared each time her depressive illness has returned. My survey revealed a number of patients for whom I had doubts about making a definite diagnosis of affective psychosis. These included two severely retarded females aged 42 and 45 at Stoke Park Hospital, who showed persistently boisterous, over-active, noisy and occasionally aggressive and destructive behaviour, with a predominantly elated mood suggestive of chronic mania. In the elder patient, the highest re corded IQwas 29, and in the younger—22, but the verbal facility of the latter suggested that her IQ must have been higher before her admission 13 years previously. Her behaviour had become more unmanageable on lithium, and both she and the other patient had shown only a minimal beneficial response to a variety of psychotropic drugs. A male patient at Stoke Park aged 32, with limited but intelligible speech, and with a highest recorded IQ of only 20,
had
a number
of features
in
common
with
the two female patients and presented a similar diagnostic problem. I felt able to make a confident diagnosis of schizophrenic illness on the basis of the usually accepted clinical criteria in the case of 15 males and 27 females—an even more marked reversal of the usually accepted sex distribution than that for manic-depressive psychosis, which I am at present unable to explain. These figures represent a prevalence rate for the Stoke Park Group as a whole of 34 per thousand (24 per thousand for males and 40 per thousand for females), very similar to Reid's figure for Strathmartine, but again there are wide variations between the prevalence rate for Stoke Park and that for each of the other three hospitals—Stoke Park 50 per thousand, Pur down 15 per thousand Leigh Court 53 per thousand and Hanham Hall 15 per thousand. The more obvious mental disturbance in
530
PSYCHOSIS IN MENTAL
schizophrenia, and its more or less ever-present nature, makes it less likely to be overlooked by medical or nursing staff than an episodic illness like manic-depressive psychosis, in which the last attack may have occurred some years before. Assuming, therefore, that my diagnosis was correct in each case, I think that the true prevalence of schizophrenic illness in the Stoke Park Group and at Strathmartine Hospital is likely to be little, if at all, higher than the figures quoted by Reid and myself. A diagnosis of hebephrenic schizophrenia was made for 10 males and 13 females. The age at onset appeared to be in the second or third decade in both sexes. In 2 male and 4 female cases the onset had been before the age of i6 years. The highest recorded IQ was in the range of 42—69in males, with the exception of one patient with an IQ of approximately 20, and in females it was in the range of 28—70, except that one patient had an IQ of 23; this patient had, however, been reported earlier as being able to read. This again illustrates the difficulty of establishing an accurate pre psychotic IQ in a case of schizophrenia seen for the first time a considerable period after the onset of the disorder. A case in point is that of Christopher, who was almost
@
15 when
he came to
Purdown in 1959 with a highest recorded IQof 58, but who is now able to express concepts which suggest that his IQ must previously have been considerably higher; it is now 51. A positive family history of psychosis was obtained in only male and 6 female patients. The most interesting concerned Jimmy, whose maternal grandmother and maternal uncle had suffered from serious psychotic illnesses, and whose younger sister, Jackie, was admitted to Stoke Park Hospital two years after her brother, with profound mental retardation but with nothing to suggest a psychosis. On admission, Jimmy's IQ was 42, whereas Jackie was only at the 6—cjmonth stage of development on the Griffiths Scale when she came to us. It is interesting to speculate that, whatever the nature of the schizophrenic process may eventually prove to be, its presence at birth may, as in the case also of the inborn errors of metabolism, inhibit almost completely normal mental development so as to produce a condi
HANDICAP
lion of profound mental retardation, indis tinguishable, as in Jackie's case, from that of many others with a similar degree of mental handicap. The frequent occurrence of autistic and non-specific psychotic features, in children with phenylketonuria and other inborn meta bolic errors, seems to add support to such speculation. Five male and 4 female patients were diag nosed as suffering from catatonic schizophrenia, on the basis of recurrent periods of extreme psychotic excitement and catatonic stupor with flexibilitas cerea, with intervals of more or less normal behaviour in between. I felt able to make a diagnosis of paranoid psychosis in i z female cases but in no males. Three of the patients had systematized delusions. The age of onset ranged from the fourth to the seventh decade, but in 5 cases it was in the fifth decade, which raises the possibility of the menopause as an important aetiological factor which might partially explain the prepon derance of female cases. Similarly, the highest recorded IQ showed a wide variation from 26 to 72, but again the patient with the IQ of 26 has a verbal facility which suggests a previously higher IQ, and the possibility that she would be more correctly diagnosed as suffering from hebephrenic schizophrenia, in spite of the absence of any definite evidence in her history of psychosis before the fifth decade. In 3 patients with paranoid psychosis there were signs of superimposed senile dementia. One patient in the paranoid psychosis group had an XO/XX chromosome mosaic comple ment. She was also deaf and blind from retinitis pigmentosa, and had suffered from oro-facial dyskinesia since 5960. Deafness was also present in another patient in this group (who suffered from congenital syphilis also), and in one male patient in the catatonic group. Epileptic fits had occurred in one male and in 2 females in the hebephrenic and one male in the cata tonic group. One male in the hebephrenic group suffered from congenital syphilis also, and another male in the same group was diabetic. Christopher suffered from eczema, and Jimmy from psoriasis, and in both cases the condition of their skin became worse during acute phases
ALAN HEATON-WARD
of their hebephrenic illness. Both were referred to by DrJancar in his Blake Marsh Lecture two years ago, when he reminded us of the greatly increased risk of further severe and irreversible brain damage that patients with such disorders run from vaccination. As mentioned previously, Reid found some evidence that the schizophrenic psychoses appeared to run a more benign course in the mentally handicapped than in patients with higher intelligence. Unfortunately, the results of up-to-date psychological assessment were not available in all cases in my survey, but the most recent assessment showed a fall in IQ of over 20
per
cent
in
5
patients
in
the
hebephrenic
group and in one patient in the catatonic group and one in the paranoid group, apart from the 3 patients already referred to as showing signs of superimposed senile dementia. Patients in both the schizophrenic psychosis and paranoid psychosis groups had been treated with a variety of phenothiazine, butyrophenone and other tranquillizers, with at least temporary symptomatic relief in most cases. Four hebe phrenic patients and one with paranoid psy chosis each received one or more courses of ECT. One male hebephrenic patient had received 33 deep insulin comas before his admission to Stoke Park, and had on one occasion developed irreversible coma and had been unconscious for two days. There were a number of other patients with a history of isolated paranoid behaviour, whose condition, I felt, did not justify a diagnosis of paranoid
psychosis.
I found no difficulty in identifying patients with pseudo-psychosis, whose apparent delu sions were more like childhood fantasies, and I did not include them in either the schizo phrenic psychosis or the paranoid psychosis group. Among these patients were Sidney, with an IQ of only 33, who could neither read nor write and who had been with us ever since he was admitted 52 years ago at the age of 14. He gave an enthusiastic, if somewhat im probable, account of his service in each of the armed forces in turn, claiming to have captained a ship in the Royal Navy. Another was Bobby, a very cheerful, sociable mongol out-patient aged 31, who had caused some anxiety at the
53' training centre which he attended by recounting his conversations with ‘¿his boys'; these turned out to be the television characters Randall and Hopkirk whom he had incorporated into his child-like fantasies and with whom he held imaginary conversations each day. My review of the histories of each of the adult patients at Stoke Park revealed a number who had one or more confusional episodes, most frequently associated with a febrile illness, but in one case with the development of classical signs of pellagra which was successfully treated with nicotinamide. There are at present 129 patients in the Stoke Park Group aged 70 and over, including 24 over
8o
and
one
I was particularly and Richards
over
90.
During
my
survey,
struck as both Reid (@@‘@)
(i@7@) had
been earlier
by how
few appeared to be showing the more obvious signs of senile dementia, such as confusion, memory loss, reversal of sleep patterns, wander ing, and deterioration in personal habits and hygiene. This was particularly striking in our 8o-plus group of old ladies, whose IQ's were mostly in the 40 to 50 range. It would seem that, contrary to my earlier impression, the signs of senile dementia do not necessarily appear earlier in the moderately and mildly retarded than in the general population, but perhaps this is true only of mentally handicapped patients cared for in an environment in which they are protected from the strains and stresses of everyday life in the community and from the self-neglect to which old people living on their own are prone. As a result of this survey, I found myself wondering why psychosis occurs in some mentally handicapped patients and not in others of the same level of intelligence, and whether the same aetiological factors apply to them
as to people
of normal
intelligence.
Of
all the autosomal chromosomal abnormalities associated with mental handicap, trisomy 21 is the one in which psychoses have most fre quently been reported by various authors, including Roith (1961, psychotic depression), Earl (@3@) and Rollin, (1946, catatonic psy chosis) and Payne (1968, paranoid psychosis). There is a great excess of cases of trisomy 21 over the number of all the other known chromo
PSYCHOSIS IN MENTAL HANDICAP
532
some abnormalities in the mentally handi capped combined, and there is at present no clear evidence that the prevalence of psychosis in mongols is, in fact, higher than that in sub jects with other chromosomal abnormalities or in the general population. However, several authors, including Reid (i@7@) and Richards (1974),
have
commented
on
the
particular
lia
bility of patients with Down's syndrome to under go premature ageing and dementia. We have one such patient, now aged 64 at Stoke Park, who has shown progressive mental deterioration of a senile type over the past six years, im mediately following an operation for cataract. On the other hand, psychoses have been reported in a number of mentally handicapped patients with a variety of abnormalities of sex chromosomes, in particular in males with extra X chromosomes. As Professor Forssman said in his 5970 Blake Marsh Lecture, ‘¿Itmay be considered established thatmen havingmore than one X chromosome are more subject to func tional psychosis than are men with only one Y chromosome.' It seems possible that sex has, in fact, more to do with mental illness than even Freud realized! In 1972, Mendlewicz and his colleagues re ported 7 families in New York in whose male members the liability to develop manic depressive psychosis seemed to go with the presence of the X-linked colour blindness gene. More recently, interest has been revived in Renpenning's syndrome, first reported in Canada in 1962 and 1963, since evidence has accumulated that this X-linked form of mental retardation is relatively common. It has been claimed by Turner (i@7i) that as many as io per cent of all mentally retarded males in the 30—35IQ range are in fact cases of Ren penning's syndrome. The physical appearance of affected persons is normal, and to date no constant biochemical abnormalities have been identified in them. As far as I know, there are no reports of the association of colour blindness or manic-depressive psychosis with Ren penning's syndrome, and I think that this would provide a fertile field for research—it would be fascinating if this association could be demonstrated and a link established between Mendlewicz's and Renpenning's cases.
The present Chairman of the Mental Defi ciency Section, Dr Valerie Cowie (1968), and other authors, including Stern (1968) and Winokur (1974), have discussed the association between a number of inborn errors of meta bolism, such as cystathionuria, homocystinuria and Hartnup disorder, and the psychoses, including schizophrenia, and the possibility of their giving clues to the biochemical mecha nisms of thought disorder—yet another ob viously fertile field for research. I feel very strongly that the staff of a mental handicap hospital should be capable of con tinning to care themselves for all their patients who develop mental illnesses, but I agree with Reid that junior staff have often had little, if any, psychiatric training and that training in clinical psychiatry, psychopharmacology and the techniques of ECT are necessary aspects of the training programmes for both medical and nursing staff. I am very concerned at the attitude of those of our medical and nursing colleagues in the mental handicap field who take the view that patients who develop a superimposed mental illness should be transferred for treatment to a mental illness hospital. In so doing, I think that our medical colleagues are calling in to question any claim they may have to be considered psychiatrists, and are reinforcing the case of those who deny the existence of the specialty of mental handicap to which Blake Marsh devoted his working life; and similarly our nursing colleagues who take this view are dangerously ‘¿jay walking' and are strengthening the case of those who would abolish the present register of nurses of the mentally subnormal and hand over the nursing care of our patients to a new caring profession. I hope I have succeeded in my aim of demonstrating that mental handicap does belong to psychiatry and that it contains a wealth of psychiatric material, as yet not fully explored, which may provide essential clues to our understanding of psychotic illness through out the full range of human intelligence. REFERENcES Cit@rr, M. (i@7i) A North Wales experiment in sub normality care. British Journal of Psychiatry, i i8, 199—206.
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